Grand Rounds - SUNY Downstate Medical CenterOur Patient HPI: 41 year old male, first visit here,...

Grand Rounds Edward Chay

SUNY Downstate Medical Center

Ophthalmology

January 16, 2014

Our Patient HPI: 41 year old male, first visit here, complaining of

blurry vision at night for the past 2-3 weeks.

- Previously seen elsewhere for poor vision 3 years

ago, told that “he has fluid in the left eye” but

reports vision had been better in the interim.

- Denies trauma, excessive lacrimation, flashes,

floaters or curtains.

- Of note, he has a month history of intermittent

parotid gland swelling, elevated ACE levels, and

hilar adenopathy on CXR. He is scheduled by ENT

for parotid gland biopsy.

Core Competency: Patient Care

Our Patient • PMH: HTN

• PSH: none

POH: self-reported poor vision OS 3 years ago, no

refractive error, surgery or trauma

• FH: no blindness or glaucoma

• SH: denies smoking, drugs, illicits

• Meds: HCTZ

• Gtts: none

• Allergies: NKDA


Our Patient Vitals: BP ranges from 130s-150s / 70s-90s

Exam:

DVasc: 20/20 OD, 20/100-2 PH 20/60 (nasal letters only) Pupils: 4 to 2 OU, no apd EOM: full OU CVF: full OU Tapp: 10, 11 @ 1:20 pm SLE: LLA: wnl OU C/S: w/q OU K: clear OU AC: deep and quiet OU I/P: round and reactive OU L: clear OU


Our Patient

OD OS


Next Step

OD OS


Further workup?

Review of Ancillary Tests • B-scan: symmetric choroidal thickness

• Labs: ACE 185 (8-52)

• CT Chest: mediastinal adenopathy with 1.2cm R

paratracheal node and 1.1 cm node anterior to

carina

• Bronchial Biopsy: RLL: multiple non-necrotizing

granulomata

• Parotid Gland Biopsy: unable because swelling

resolved


Goals

• Periphlebitis o Differential diagnosis

• Sarcoidosis: Posterior Segment o Presentation

o Epidemiology

o Findings

o Treatment

o Prognosis

Differential Diagnosis Periphlebitis

• Panuveitis o Sarcoidosis

o Behcet’s

o Tuberculosis

o Sympathetic Ophthalmia

o VKH

• MS

• Infectious o ARN, Toxo, CMV

• Leukemia

Unilateral Neurosensory Detachment

• CSCR

• Uveitis

• RD

• CNV

Core Competency: Medical Knowledge


• Panuveitis o Sarcoidosis o Behcet’s

o Tuberculosis


o VKH

• MS


• Leukemia


• CSCR • Uveitis

• RD

• CNV


Unifying Diagnosis?


• Panuveitis o Sarcoidosis o Behcet’s

o Tuberculosis


o VKH

• MS


• Leukemia


• CSCR

• Uveitis • RD

• CNV


Unifying Diagnosis?

Retinal Vasculitis • Starts with

o Breakdown of inner blood-retina barrier (endothelial cell junctions)

o Diapedesis of inflammatory cells

• Leads to o Continued leak -> edema, or

o Occlusion -> neovascularization and fibrosis

• “Healing phase” o Gliosis of vascular walls (venous sheathing)

o 4 types of peripheral venous sheathing:

• Congenital sheathing (persistent embryonic connective tissue)

• Post-inflammatory sheathing (perivascular gliosis)

• Halo sheathing (venous sclerosis)

• Periphlebitic sheathing (active periphlebitis)


Retinal Vasculitis • Panuveitis

o Sarcoidosis

o Behcet’s

o Tuberculosis

o Sympathetic

o VKH

• MS


• Leukemia



o Sarcoidosis

o Behcet’s

o Tuberculosis

o Sympathetic

o VKH

• MS


• Leukemia


FA: diffuse microvascular leakage found in 100% of cases

Photo credit: Differential Diagnosis of Retinal Vasculitis


o Sarcoidosis

o Behcet’s

o Tuberculosis

o Sympathetic

o VKH

• MS


• Leukemia


FA: Most common retinal finding is periphlebitis Usually a presumed diagnosis based on symptoms and CXR findings



o Sarcoidosis

o Behcet’s

o Tuberculosis

o Sympathetic

o VKH

• MS


• Leukemia


No history concerning for rupture or previous PPV


o Sarcoidosis

o Behcet’s

o Tuberculosis

o Sympathetic

o VKH

• MS


• Leukemia


No history consistent with prodromal, acute, convalescent, or recurrent phase


o Sarcoidosis

o Behcet’s

o Tuberculosis

o Sympathetic

o VKH

• MS


• Leukemia


FA: patchy perivascular cuffing indicates activity, wall whitening with no leakage represents chronic sclerotic change



o Sarcoidosis

o Behcet’s

o Tuberculosis

o Sympathetic

o VKH

• MS


• Leukemia



Sarcoidosis o Epidemiology

• Affects all ethnic groups, highest prevalence in northern Europeans

• In US, affects AA > white

• ACCESS study: workplace exposure to mold, musty odors, insecticides

is associated with increased risk of sarcoidosis

• HLA-DRB1

• Mycobacterial organisms may be etiologic factors


Sarcoidosis o Pathology

• Noncaseating granuloma

• Sarcoid tubercle: epithelioid cells, Langhans, rim of lymphocytes

o Systemic Manifestations

• Lofgren syndrome

• Heerfordt syndrome

• Acute and chronic forms


Sarcoidosis o Ocular Manifestations

• Orbital, lacrimal, conjunctival, corneal, glaucomatous, iris

• Anterior uveitis found in 67% of patients

• Intermediate uveitis

• Posterior uveitis found in 25-30%

o Macular edema: most common sight-threatening consequence

of sarcoid uveitis

o Neovascularization is most significant factor contributing to poor

prognosis [Spalton et al. 1981]

o Sequelae include vitreous hemorrhage, ischemia, and retinal

detachment

• Neuro


Posterior Sarcoid Uveitis o 1/3 have no anterior findings! [Wertheim 2005]

o Active periphlebitis: fluffy white haziness around vessels

o Candle-wax drippings:

• segmental venular cuffing, sheathing and perivenous exudation

• non-necrotising granulomas

• not pathognomic for sarcoid! (syphilis, TB, Behcet’s)

o 15% of posterior uveitis develop neovascularization

o FA: venule wall staining, focal leakage, capillary closure, cystoid macular

edema, neovascularization

Sarcoidosis o Diagnosis

• Screening:

o CXR: 90% sensitivity, thin-cut spiral CT is more sensitive

o ACE, lysozyme are better for tracking active disease (not

diagnostic or specific)

o Positive gallium + ACE elevation + uveitis is highly specific

• Vitreous fluid analysis

o vitreous tap and immunological analysis

o CD4/CD8 > 3.5: sensitivity of 100% and a specificity of 96.3%.

• Diagnosis ultimately made with biopsy


Sarcoidosis o Treatment

• TB skin test

• Anterior uveitis:

o topical steroids (Pred Forte 1% > generic > prednisolone

phosphate) + cycloplegia

o Lotemax; Vexol and FML good for maintenance

• Vision threatening posterior uveitis:

o Systemic corticosteroids (prednisone 40-80 mg/day)

o Intravitreal fluocinolone acetonide implant

• Immunomodulation therapy

• PRP

• Surgery

o cataract surgery risks much higher with anterior uveitis

o Trabeculectomy or glaucoma drainage device

o Vitrectomy


Vitrectomy for Sarcoid • Inconclusive sarcoid diagnosis [Matsuoka, Clin Ophthal 2012]

o Case reports

o Epithelioid and multinucleated giant cells pathognomic for

sarcoid

• Vitreous opacity [Ieki et al, Ocul Immunol Inflamm. 2004]

o 11 eyes with vitreous opacities and uveitis associated with

sarcoidosis resistant to corticosteroid therapy

o 7 gained 2 or more lines of Snellen VA 6 months postoperatively

• Epiretinal membrane [Kiryu, JPn J Ophthalmol. 2003]

o 11 eyes with epiretinal membrane and uveitis with sarcoidosis

underwent pars plana vitrectomy

o 9 gained 2 or more lines of Snellen VA 1-12 months after surgery;

however 4 of these lost 2 or more lines by final visit 2/2 cataract

formation and membrane reformation

Sarcoidosis o Prognostic factors

• Chronic posterior uveitis, glaucoma, delay in presenting to uveitis

specialist for > 1 year, presence of intermediate or posterior uveitis

associated with visual loss

Returning to our patient • Multiple visits over the last 3 years

• VA improved from 20/100-2 to 20/60, neuroretinal

detachment resolved, no sub-retinal fluid

• Course involved no steroids (minimal inflammation)

• Still following closely


2 years later On presentation

Reflective Practice • This case represented application of careful history

taking, ophthalmic examination and creation of a

complete differential diagnosis to evaluate and

treat a complex retinal disorder.

Core Competencies —Patient Care: The case involved thorough patient care and careful attention to the

patient's past medical history.

—Medical Knowledge: This presentation allowed me to review the presentation,

differential diagnosis, proper evaluation/work up, treatment options for sarcoidosis.

—Practice-Based Learning and Improvement: This presentation included a literature

search of current diagnostic and treatment modalities.

—Interpersonal and Communication Skills: The patient was made aware of the

complexity of the diagnosis and is actively engaged

—Professionalism: The patient provided information for the grand rounds presentation

only after careful exam, explanation of findings and use of information.

—Systems-Based Practice: The patient’s history was obtained through multiple health

care deliverer’s (PMD, ENT, ophthalmology)

References

• BCSC Intraocular Inflammation and Uveitis

• Kanski

• Walsh and Hoyt. Clinical Neuro-Opthalmology

• Pleyer and Forrester. Uveitis and Immunological Disorders: Progress III

• El-Asrar, Herbort, Tabbara. Differential Diagnosis of Retinal Vasculitis. Middle East Afr J Ophthalmol. 2009 Oct-Dec; 16(4): 202-218

• Wertheim MS, Mathers WD, Suhler EB, Wilson DJ, Rosenbaum JT. Histopathological features of conjunctival sarcoid nodules using noninvasive in vivo confocal microscopy. Arch Ophthalmol. Feb 2005;123(2):274-6

• Spalton DJ, Sanders MD. Fundus changes in histologically confirmed sarcoidosis. Br J Ophthalmol. May 1981;65(5):348-58

• Matsuoka M et al. Two cases of ocular sarcoidosis in which vitreous cytology was useful for supporting the diagnosis. Clinical Opthalmol. 2012; 6:1207-9

Acknowledgements • Dr. Kenneth Olumba

• Dr. Joseph Tseng

Grand Rounds - SUNY Downstate Medical CenterOur Patient HPI: 41 year old male, first visit here,...

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